Pain and Sensation

You may be relieved to know that the muscle deterioration in Duchenne and Becker isn't usually painful in itself. Some people report muscle cramps at times; these can usually be treated with over-the-counter pain relievers.

Also, since muscular dystrophy doesn't affect nerves directly, those who have the disorders retain normal sensations of touch and other senses. They also usually have control over the smooth, or involuntary, muscles of the bladder and bowel, and have normal sexual functions.

The Heart

Like muscles in the limbs, heart muscle also can be weakened by lack of dystrophin. By the teen years, the damage done by DMD to the heart can become life-threatening. This system should be monitored closely, usually by a pediatric cardiologist.

People with DMD and BMD often develop cardiomyopathy — heart muscle weakness — because of a deficiency of dystrophin. The muscle layer (myocardium) of the heart deteriorates, just as the skeletal muscles do, putting the person at risk of fatal heart failure.

Some people with BMD have mild skeletal muscle involvement but severe cardiac problems.
In 2005, the American Academy of Pediatrics released its recommendations for people with DMD and BMD and carriers of these diseases.

In 2005, the American Academy of Pediatrics released its recommendations for people with DMD and BMD and carriers of these diseases.

The academy recommends that those with DMD have a complete cardiac evaluation by a specialist beginning in early childhood and again at least every other year until age 10. After that, the evaluations should be done every year or at the onset of symptoms of heart weakness, such as fluid retention or shortness of breath.

For those with BMD, it recommends evaluations at least every other year beginning at age 10.

Carriers of DMD and BMD are at higher than average risk of developing cardiomyopathy. The academy suggests that carriers should undergo a complete cardiac evaluation in late adolescence or early adulthood, or sooner if symptoms occur, and that they should be evaluated every five years starting at age 25 to 30.

There’s some evidence that treatment with angiotensin converting enzyme (ACE) inhibitors and beta blockers can slow the course of cardiac muscle deterioration in DMD and BMD.
This is effective if the medications are started as soon as abnormalities on an echocardiogr-am (ultrasound imaging of the heart) appear but before symptoms occur.

Some people with BMD who have severe heart problems but generally good health have been successfully treated with heart transplants.

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Respiratory Function

After a boy with DMD is about 10 years old, the diaphragm and other muscles that operate the lungs may weaken, making them less effective in moving air in and out. Problems that may indicate poor respiratory function include headaches, mental dullness, difficulty concentrating or staying awake, and nightmares.

Anyone with a weakened respiratory system is also subject to more infections and difficulty in coughing. A simple cold can quickly progress to pneumonia in this person. During infections, it's important to get prompt treatment before a respiratory emergency occurs.

As breathing ability declines, the family can get a coughing machine or learn procedures to assist with coughing and keep the bronchial system free from secretions. A respiratory therapist or pulmonologist can be consulted for the needed information.

At some point, assisted ventilation may be needed to help provide sufficient air flow into and out of the lungs.

The first step in using assisted ventilation is usually a noninvasive device, meaning one that doesn’t require any surgical procedures. The person receives air under pressure through a mask, nosepiece or mouthpiece. Later, if full-time ventilatory support is needed, some young men switch to an invasive system, which means that a surgical opening called a tracheostomy is performed, allowing air to be delivered directly into the trachea (windpipe).

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MDA’s booklet “Breathe Easy: Respiratory Care in Neuromuscular Disorders” gives detailed information on this topic.

Intellectual Effects

About a third of boys with DMD have some degree of learning disability, although few are seriously retarded. Doctors believe that dystrophin abnormalities in the brain may cause subtle cognitive and behavioral deficits. The learning problems seen in some people with DMD and BMD occur in three general areas: attention focusing, verbal learning and memory, and emotional interaction.

Children suspected of having a learning disability can be evaluated by a developmental or pediatric neuropsychologist through the school system's special education department or with a referral from the MDA clinic. If a learning disability is diagnosed, educational and psychological interventions can begin right away. The specialist may prescribe exercises and ways to interact with your child that can help improve these deficits, and the school can also provide special help with learning.

 

CAN SPECIAL DIETS OR EXERCISES HELP IN DMD AND BMD?

Diet

Many people, when they hear the words “lack of a protein,” logically ask, “Should I eat more protein?” Unfortunately, eating more protein has no effect on any of the proteins missing in muscular dystrophy.

No special dietary restrictions or additions are known to help in DMD or BMD. Most doctors recommend a diet similar to that for any growing boy, but with a few modifications.

A combination of immobility and weak abdominal muscles can lead to severe constipation, so the diet should be high in fluid and fiber, with fresh fruits and vegetables dominant.

For boys who use power wheelchairs, take prednisone or who aren't very active, caloric intake should probably be somewhat restricted to keep weight down. Obesity puts greater stress on already weakened skeletal muscles and the heart. Doctors have found that a low-calorie diet doesn't have any harmful effect on the muscles.

Those on prednisone and those with heart problems also may need a sodium-restricted diet.

Exercise

Exercise can help build skeletal muscle, keep the cardiovascular system healthy, and contribute to feeling better. But in muscular dystrophy, too much exercise could damage muscle. Consult with your doctor about how much exercise is best. A person with DMD or BMD can exercise moderately but shouldn't go to the point of exhaustion.

Some experts recommend swimming and water exercises (aquatic therapy) as a good way to keep muscles as toned as possible without causing undue stress on them. The buoyancy of the water helps protect against certain kinds of muscle strain and injury. Before undertaking any exercise program, make sure you've had a cardiac evaluation.

Physical and Occupational Therapy

A physical therapy program is usually part of the treatment for DMD and BMD. Your MDA clinic physician will refer you to a physical therapist for a thorough evaluation and a personal exercise program.
The primary goals of physical therapy are to allow greater motion in the joints and to prevent contractures and scoliosis.

Occupational therapy focuses more on specific activities and functions, while physical therapy emphasizes mobility and, where possible, strengthening of large muscle groups. OT can help with tasks related to work, recreation or daily living, such as driving, dressing or using a computer.


Facts About Duchenne and Becker Muscular Dystrophy booklet from MDA

 
 
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